David F. Fretzin

Atypical fibroxanthoma of the skin. A clinicopathologic study of 140 cases

In an attempt to further understand the nature of atypical fibroxanthoma of the skin, 140 lesions were subjected to clinical, histologic, and histochemical studies. Atypical fibroxanthoma most commonly presented as a solitary, nonspecific nodule or ulceronodule on exposed skin of the face in the elderly. A clinical variant occurred in much younger persons on the covered areas of the trunk and limbs. Histologically, atypical fibroxanthoma develops as a circumscribed, cellular proliferation within the dermis, with occasional infiltration into the subcutis. Morphological patterns vary from lesions showing plump spindle cells in interlacing fascicles to those with haphazardly arranged large polyhedral cells. Bizarre multinucleated giant cells with foamy cytoplasm and numerous mitotic figures enhance its distrubing sarcoma‐like appearance. Among 101 patients followed for periods up to 15 years, no metastatic lesions were found and only nine lesions recurred. Correlation of the clinical and follow‐up data support the concept that in spite of its alarming histologic appearance, atypical fibroxanthoma of skin appears to behave in a benign manner.

Disseminated trichosporon beigelii (cutaneum)

Two cases of invasive Trichosporon beigelii (syn. cutaneunt) infection are reported and are compared with the eight other previous reports. All affected patients were either immunosuppressed or had recently undergone a surgical procedure. The diagnosis had been delayed and the prognosis was poor. Only two patients recovered after vigorous antimycotic therapy and concomitant remission of their leukemia. A biopsy of the skin lesion, as illustrated in one of our patients, may prove to be useful in the early diagnosis.

Clear cell basal cell carcinoma: An unusual degenerative variant

Clear cell basal cell carcinoma (BCC) is an unusual variant of BCC which is characterized by a variable component of large clear cells. Twenty cases are presented which are further subdivided into pure, mixed, or metatypical categories. Although initially thought to represent sebaceous or tricholemmal differentiation, electron microscopic studies demonstrated that the clear cell changes are a degenerative phenomenon, probably involving lysosomes.

Angiolymphoid hyperplasia demonstrating extensive skin and mucosal lesions controlled with vinblastine therapy

Since Kimuras original description of an unusual subcutaneous disorder with distinctive histologic features of vascular and lymphoid proliferation, numerous descriptions of diseases with similar features have been reported. A variety of descriptive labels, including Kimuras disease, angiolymphoid hyperplasia (ALH) with eosinophilia, atypical pyogenic granuloma, papular angioplasia, and histiocytoid hemangioma, have been applied to these diseases. Although this information has broadened our understanding of the clinical and pathologic spectrum of ALH, the etiology and/or pathogenesis remains unknown. Our case report illustrates the wide range of clinical features of ALH, demonstrating extensive lesions on skin and mucosa. The extreme histologic variability with regard to eosinophils, lymphoid infiltrates, and vascular alterations is also illustrated. Dramatic response to vinblastine sulfate treatment has not been previously reported and may provide a viable treatment alternative in selected patients.

Genetic counseling in segmental neurofibromatosis

We report two patients with segmental neurofibromatosis and review the literature with regard to possible hereditary transmission of this disorder. Patients that meet strict criteria for the diagnosis of segmental neurofibromatosis seem to have a low probability of transmitting the disease. We emphasize the importance of establishing and strictly adhering to a set of diagnostic criteria and of obtaining a comprehensive family history when reporting cases of segmental neurofibromatosis.

Congenital pilar and smooth muscle nevus

A case of congenital pilar and smooth muscle nevus (CPSMN) is reported. CPSMN is a distinct clinical and histologic entity characterized by prominent vellus hairs accompanying a proliferation of smooth muscle within the reticular dermis. Plaquelike elevation and mild hyperpigmentation are variable features of this unusual organoid nevus.

Bronchial carcinoid metastatic to skin. Light and electron microscopic findings.

This case report illustrates the value of ultrastructural examination of an undifferentiated carcinoma metastatic to skin. In this patient, ultrastructural study of a cutaneous nodule demonstrated cytoplasmic neurosecrctory granules characteristic of an amino precursor‐uptake and decarboxylation cells (APUD) tumor and supported the diagnosis of metastatic bronchial carcinoid. Additional nine cases of bronchial carcinoids metastatic to skin are also briefly reviewed.

Woolly-Hair Nevus: A Case Report and Study by Scanning Electron Microscopy

Abstract: Woolly‐hair is a congenital defect that produces a localized patch of lightly colored, curly scalp hair. Scanning electron microscopy of the abnormal hairs demonstrates twisting of the hair shaft and abnormal cuticle formation.

Eccrine syringofibroadenoma. A clear-cell variant.

Eccrine syringofibroadenoma (acrosyringeal nevus) is a rare tumor of proliferating ductular structures resembling the acral portion of the eccrine duct. Our case describes a histologic variant showing nests of periodic acid Schiff-positive clear cells resembling the clear-cell variant of eccrine syringoma.

Pheomycotic cyst in an immunosuppressed host

Abstract A case of pheomycotic cyst due to Exophiala jeanselmei is reported in a patient having chronic lymphocytic leukemia and systemic lupus erythematosus. Isolation and identification of the organism causing pheomycotic cyst is important in terms of prognosis and treatment of this condition.